Cutaneous T-cell lymphoma

Cutaneous T-cell lymphoma

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Key Points *Malignancy of the lymphatic system that is localized to the skin in most cases *Exact cause is unknown, but may be associated with other skin conditions or retrovirus infection

Cutaneous T-cell Lymphoma (CTCL) is an uncommon condition in which the abnormal neoplastic formation of lymphocytes cause a proliferation of tumors of the lymphatic system. It is typically divided into two subtypes, indolent and aggressive.

Mycosis Fungoides is the most common form of indolent CTCL, a rare condition which results in the buildup of abnormal lymphocytes. Affected persons may see years pass before the condition progresses to its next stage. Mycosis Fungoides initially consists of flat, pink lesions which may be oval or annular, and may be accompanied by itching and thinning of the skin. This is known as the patch stage of the condition. In the plaque stage, lesions thicken and are accompanied by moderate itching. The tumor stage sees the development of large lumps of irregular shape on plaques, which may become ulcerated. Metastasization becomes more likely in the tumor stage than in previous stages. There are also several symptomatic variants of Mycosis Fungoides. These include folliculotropic MF (normal progression of the disorder affecting the areas around hair follicles, typically the on the head and/or neck, sometimes accompanied by hair loss in the affected area), pagetoid reticulosis (consists of patches or plaques, usually on the extremities, caused by a buildup of neoplastic T-cells), and granulomatous slack skin (rare variant causing development of lax skin folds in areas with primary skin folds, such as the groin or armpits).

Primary cutaneous CD30+ lymphoma accounts for approximately 30% of all indolent CTCL cases. Primary cutaneous anaplastic large cell lymphoma typically consists of solitary or localized tumors, which often ulcerate, though multifocal lesions are seen in 1/5 of affected persons. Lesions may spontaneously regress, and 1/10 of affected persons will experience regional lymphatic involvement. The prognosis for this type is typically good. Lymphomatoid papulosis is a chronic, self-resolving skin disease in which lesions form on the extremitites and clear within 3-12 weeks, and may scare. Recurrences are common in this form of lymphoma.

The two aggressive forms of CTCL are particularly rare and deadly. Sezary syndrome affects the entire skin surface, causing skin to become bright red. This is often accompanied by thickening, dryness, scaling and severe itching. Sezary syndrome, due to its widespread nature, is often fatal, with affected persons typically subject to severe infection stemming from immunosuppression. Adult T-cell lymphoma is a very serious blood disease in which atypical cells invade the bloodstream in large numbers, and is associated with the presence of the HTLV I retrovirus. Acute ATLL is similar in appearance to MF and Sezary syndrome, and accompanied by enlarged lymph glands, increased calcium levels and bone lesions. Prognosis of this type is particularly poor, ranging from two weeks to one year. Chronic ATLL typically presents only skin lesions, but may progress into the acute form.

DiagnosisKey Points *Initial diagnosis based on characteristic appearance of the affected area *Skin Biopsy will be performed to confirm diagnosis and rule out other conditions

CTCL is initially diagnosed based on the characteristic appearance of the affected area. A skin biopsy will be performed to confirm the diagnosis and to rule out other, similar appearing conditions. Multiple biopsies may be needed for complete diagnosis.

Treatment *Method and success of treatment is predicated on severity of the condition and available treatments *Various treatments may prove helpful in combating the condition

The method and success of treatment of CTCL is often dependent on the type or stage of the condition and the availability of treatment options. There are various treatment options which may prove helpful in combating the condition, including topical steroids, Phototherapy, PUVA Photochemotherapy, topical Nitrogen mustard, bexarotene, chemotherapy, electron beam and/or localized radiotherapy, interferons, and photopheresis.